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A Case of POEMS Syndrome
 

A Case of POEMS Syndrome

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    A Case of POEMS Syndrome A Case of POEMS Syndrome Presentation Transcript

    • PROF.DR.NOORUL AMEEN PANDICHELVAN M6
      • 40 yrs old female came with
      • c/o breathlessness -2 mon
      • insidious onset
      • progressive
      • class 2
      • related with exertion
      • h/o easy fatigability
      • h/o arthralgia
      • h/o tiredness
      • h/o loss of weight
      • No h/o cough and sputum production
      • palpitation
      • orthopnea
      • paroxysmal nocturnal dyspnea
      • chest pain
      • Past history:
      • Not know case of DM/HT/PT
      • Personal history:
      • Not an Alcoholic/Smoker
      • Menstrual history:
      • Attained menarche @ 12 yrs of age
      • Regular; once in 26 days
      • Last for 4 days
    •  
      • General examination
      • Conscious
      • Oriented
      • Pallor
      • Hyperpigmentation seen
      • Scar seen in left side neck
      • Not Icteric
      • No Cyanosis
      • No Clubbing
      • No Pedal edema
      • No Lymph node enlargement
      • BP 90/60 mmhg Pulse rate 70/min
      • CVS : S1S2 heard
      • P2 loud
      • RS : NVBS
      • BAE
      • No added sound
      • P/A: Soft
      • CNS: NFND
      • Provisional diagnosis
      • Pulmonary hypertension
      • ? Addison disease
      • ? MCTD
      • INVESTIGATION
      • Urine examination
      • Albumin- nil
      • Sugar - nil
      • Deposit- nil
      • CBC
      • Hb -9.2 TC -5700
      • DC - P60 L40 E0 PCV -33
      • ESR -6/15 PLC -1.6 lakh
      • MCV -86
      • BLOOD Urea 36
      • Sugar 106
      • Creatinine 0.8
      • Sodium 130
      • Potasium 3.9
      • ECG –normal
      • CXR - Cardiomegaly
      • Echocardiography
    •  
    •  
    •  
      • BLOOD IMMUNOLOGY
      • ANA Positive 1:40 dilution
      • CRP Negative
      • ASO Negative
      • RF Negative
      • Hypothyroidism
      • Pulmonary hypertension
      • Anemia
      • ? Cause
      • Patient treated with
      • Tab. Thyroxine 100 ug od
      • Tab. Deriphylline tds
      • Tab. Bct tds
      • Patient advised to do
      • ACTH stimulation test
      • After two months patient comes with
      • c/o breathlessness
      • c/o abdominal pain
      • c/o athralgia
      • c/o numbness in both lower limbs
      • c/o hard of hearing
      • On examination
      • Conscious
      • Oriented
      • Pallor
      • Not Icteric
      • No Cyanosis
      • No Clubbing
      • B / L Pitting pedal edema
      • No Lymphnode enlargement
      • CVS: S1S2 heard
      • P2 loud
      • RS: NVBS
      • BAE
      • No Added sound
      • P/A: Abdomen distension
      • Moves with respiration
      • Flanks full
      • Shifting dullness +
      • CNS: HMF Normal
      • CN Normal
      • TONE Normal
      • POWER RT LT
      • UL 4 4
      • LL 4 4
      • DTR Present Present
      • B/L Plantar Flexor
      • Sensory system – Vibration sensation lost in both lower limbs
      • Cerebellar and autonomic nervous system- Normal
      • Hypothyroidism
      • Pulmonary hypertension
      • Ascities
      • Periperal neuropathy
      • Anemia
      • Cause - ? MCTD
      • ? Autoimmune polyglandular syndrome
      • INVESTIGATIONS
      • Urine Examinations
      • Albumin-1+
      • Sugar-nil
      • Deposit – 4 to 5 pus cells/hpf
      • Renal lab values
      • Glucose – ve
      • Bilirubin neg
      • Ketone neg
      • Specific gravity 1.010
      • Blood 2+
      • ph 5.0
      • Protein 2+
      • Nitrogen neg
      • Leucocytes neg
      • 24 hr urine protein- 20mg/day
      • COMPLETE BLOOD HAEMOGRAM
      • HB-11.5
      • RBC-3MILLION/CUMM
      • ESR-10/22
      • MCHC-30.1
      • MCV-79.4
      • MCH-28.4
      • PCV-31
      • PLT-1.2
      • TC-5200
      • DC-P69;L30;E1
      • BLOOD Sugar-105mg FBS-59MG/DL; PPBS-123MG/DL
      • Urea-71mg
      • Creatinine-1.5mg
      • Sodium-135 meq/l
      • Potasium-5.1meq/l
      • Chloride-108 meq/l
      • LIVER FUNCTION TEST
      • Total bilirubin-0.8
      • SGOT-18
      • SGPT-16
      • ALP-78
      • Total protein-5.9
      • Albumin-3.9
      • ABG ANALYSIS : ph-7.38
      • po2-85.4mmhg
      • pco2-27.6mmhg
      • hco3-16.2mmol/l
      • h+-41.2nmol/l
      • na-131.3mmol/l
      • k-5.50mmol/l
      • ag-18.9mmol/l
      • Absolute eosinophil count-80 cells/cumm
    • ASCITIC FLUID ANALYSIS Protein-4.4 Sugar-92 Aalbumin-1.2 SAAG -2.66 Cell count - 96%lymphocytes;4%mesothelial cells= Cytology- clusters of reactive mesothelial cells and few lymphocytes in a eosinophilic background-reactive effusion Mantouex test : Negative
      • USG ABDOMEN
      • Ascities
      • B/L increased renal cortical echos
      • Liver – normal size
      • Spleen- normal size
      • ECHO-
      • RA/RV dilated
      • TR moderate pg 46mmhg
      • Moderate pulmonary hypertension
      • No RWMA
      • Normal LT ven systolic function
    •  
      • ENDOCRINOLOGY STUDY
      • THYROID FUNCTION TEST
      • T3- 47.02ng/dl(80-200ng/dl)
      • T4- 5.77ugm/dl(4.6-12ugm/dl)
      • TSH-16.322miu/ml(0.27-4.20miu/ml)
      • Anti TPO antibodies -30.39 iu/ml(0-34iu/ml)
      • Serum Cortisol -8.32ugm/dl(6.2-19.4iu/ml)
      • ACTH stimulation test
      • serum Cortisol-8.53ugm/dl
      • DHEA sulphate-21.60ugm/dl(80-560ugm/dl)
      • Parathyroid hormone – 77.82pg/ml (15-65pg/ml)
      • Patient treated with
      • Tab.Prednisolone 5mg 6 tablets
      • Tab.Fludrocortisone 50ugm
      • Cap.Omeprazole 40mg bd
      • Tab.Eltroxine 100 mg bd
      • Tab. Calcium lactate tds
    •  
      • BLOOD IMMUNOLOGY
      • ANA- Positive 1:100 dilution
      • Ds-DNA ab Negative
      • Scl 70 ab Negative
      • Anti centromere ab Negative
      • Sm antibody Negative
      • U1 nrnp Negative
      • Blood HBsAG :Negative
      • Anti hcv: Negative
      • hiv elisa:Negative
      • vdrl Negative
      • Pulmonary
      • function
      • test
    •  
    •  
      • Ct abdomen
    •  
    •  
    •  
    •  
    •  
    •  
      • Blood biochemistry
      • Calcium-11.1 mg/dl then comes to 8.6 mg /dl
      • Phosphorus -5.1mg/dl
      • LDH-131u/l
      • ALP-104.1 iu/l
      • 24 hr urine calcium 56mg/dl
    • Differential diagnosis for metastatic sclerotic leisons
      • Thyroid carcinoma
      • Renal cell carcinoma
      • Breast carcinoma
      • Gastric carcinoma
      • Skin carcinoma
    •  
    •  
    •  
    •  
      • Nerve conduction study
    •  
    •  
      • Results:
      • Upper limb
      • Right median and ulnar nerve CMAP latency,amplitude and velocity –WNL
      • Left side - Absent
      • Lower limb
      • both CMAP AND SNAP not obtained
      • Impression
      • Both sensory motor neuropathy of both lower limb and possible sensory motor neuropathy of upper limb
      • Meanwhile patient said
      • c/o headache –B/L
      • Diffuse
      • Dullache
    •  
      • Patient developed lymphnode enlargement
      • B/L inguinal
      • B/L axillary
      • FNAC
      • Rt inguinal LN: c/c lymphadenitis
      • Left inguinal LN:material inadequate for definite opinion
      • Right axilla LN: granulomatous lymphadenitis
      • Left axilla LN: c/c lymphadenitis
      • biopsy
    •  
      • Serum electrophoresis- normal
      • protein 5.1
      • albumin 3.2
      • Urine BJP Negative
      • Serum ACE 16.3 u/l(8-52)
    •  
    •  
    •  
    •  
    •  
    •  
      • Patients have
      • hypothyroidism
      • addison disease
      • hyperparathyroidism
      • pht
      • severe restrictive lung disease
      • papilledema
      • glomerulonephritis
      • ascities
      • b/l pitting pedal edema
      • polyneuropathy
      • m protein elevation
      • sensory neural hearing loss
      • multipe osteosclerotic leison
      • castle man disease-multicentric
      • Clinically diagnosed as poems syndrome
      • Haematology opinion:
    •  
    •  
    •  
    •  
      • Final diagnosis
      • POEMS SYNDROME
      • P- polyneuropathy
      • O-organomegaly
      • E-endocrinopathy
      • M-m protein component
      • S- skin changes
      • Patient treated with
      • Tab.Prednisolone 5 mg 6 tab
      • Tab.Fludrocortisone 50 ug
      • Tab.Eltroxine 100 ug od
      • Tab.Amitriptylline 2.5 mg bd
      • Tab.Calcium lactate tds
      • Cap.Omeprazole 40 mg bd
    • Poems syndrome
      • Defined as the combination of plasma cell proliferation disorder(typically myeloma) , polyneuropathy and effects on many other organ systems
      • Peak incidence around 5-6 th decade of life unlike myeloma which is common around 6-7 th decade of life
      • Twice as common in men
      • Other names: osteo sclerotic myeloma, pep syndrome, crow fukase syndrome, takatsuki syndrome
      • DIAGNOSTIC CRITERIA
      • Polyneuropathy
      • Monoclonal plasma cell disorder
      • One or more of the following major criteria
      • - Sclerotic bone lesion
      • - Castleman’s disease
      • - Elevated VEGF levels
      • One or more of the following minor criteria
      • Organomegaly ( enlarged spleen,liver or lymphnode)
      • Extra vascular volume overload(pleural effusion,ascities,pedal edema) endocrinopathy(adrenal,pitutary,gonadal,parathyroid)dm,hypothyroidism alone insufficient
      • Papilledema
      • Thrombocytosis or Polycythemia
      • Skin changes-hyperpigmentation,dermis thickening,hirsutism,hyperhidrosis
      • Glomerulonepritis
      • Sensory neural hearing loss
      • These patients have only 5% or less than bone marrow plasma cell
      • Rarely have hypercalcemia or renal insufficiency
      • 1/3 rd to1/2 of pt have poly neuropathy where as in classic multiple myeloma only 1-8% of pts have polyneuropathy
      • Etiology
      • Caue is unknow
      • Anti body to HHV 8 seen 78% of cases with castleman disease and 22% of patients without castleman disease
      • Increased level VEGF, IL1BETA, IL6 and TNF alpha
      • VEGF targets the endothelial cells and promote angiogenesis
      • Both IL 1 beta and IL6 stimulate VEGF production
      • Elevated level of VEGF correlate with disease activity
      • Elevated VEGF accounts for organomegaly,edema and skin changes; but role in polyneuropathy uncertain
      • POLYNEUROPATHY
      • Though resembles CIDP it is motor predominance
      • Marked slowing of conduction velocity with prolonged distal latencies and progressive dispersions of compound muscle action potentials with stimulation of motor nerves proximally
      • Nerve biopsy shows combination of axonal degeneration and demyelination
      • ORGANOMEGALY
      • Hepatomegaly and Lymphnode enlargement seen in 2/3 rd of patients
      • Spleenomegaly seen in 1/3 rd of patients
      • Between 11-30% patients have castleman’s disease
      • Association is not fully understood
      • MONOCLONAL PLASMA PROLIFERATIVE DISORDER
      • Monoclonal protein is not large
      • May be missed on serum electrophoresis in 1/3 rd of patients
      • These patients require immunofixation electrophoresis of serum/urine
      • <10% patients have no circulating protein
      • They need biopsy proof of plasmocytoma
      • In general number of plasma cells are low and bone marrow shows hypercellular and reported as reactive or myeloproliferative disorder
      • SCLEROTIC BONE LESION
      • Approximately seen in 95% of patients
      • One half of patient have solitary sclerotic lesion
      • 1/3 rd of patient have multiple sclerotic leison
      • Pelvis ,spine, ribs and proximal extremites are often involved
      • PULMONARY DYSFUNCTION
      • Pulmonary hypertension,restrictive lung disease and impaired diffusion capacity of carbon monoxide may occur
      • Improvement of PHT after therapy has been reported
      • Only 19% of poems syndrome initially presented with pulmonary hypertension
      • HAEMATOLOGY
      • 50% OF cases have thrombocytosis
      • 20% of cases have mild erythrocytosis; anemia is not a feature of poems syndrome
      • May be arterial or venous thrombosis
      • TREATMENT
      • Corticosteroid-15% patients shows clinical improve ment and 7% of cases shows clinical stabilisation;should not be consider definite therapy rather temporizing therapy
      • Alkylating agents
      • Cyclophosphamide-as a single agent or in combination with prednisolone shows clinical improvement in 40% of cases
      • Melphalan- most effective agent against plasma cell disorder ; but there is risk of mds /acute leukemia
      • Thalidomide
      • Has anti vascular endothelial growth factor and anti tumour necrosis factor
      • Cause peripheral neuropathy in 20% patients receiving patients
      • Worsen the pedal edema
      • As a single agent no more effective than oral alkylators
      • RADIATION
      • patients with single or multiple sclerotic agent in limited area –treated with radiation therapy
      • Widespread sclerotic leison –systemic therapy needed
      • High dose chemotherapy and stem cell transplantation have been tried
      • Intravenous immunoglobulin and plasmapheresis shows no clinical benefit
      • Bevacizumab – monoclonal antibody against vegf have been tried
      • All trans retenoic acid has been used to suppress the production of proinflammatory cytokines
      • Prognosis
      • If untreated often fatal
      • Only 60% of patients alive 5 yrs after onset
      • Prognosis is better for patient with single sclerotic leisons
      • Multiple bone leison – intermediate prognosis
      • Common cause of death-cardiorespiratory failure,infection and capillary leak syndrome
    • THANK YOU
      • REF: HARRISON 17 EDI
      • OTM
      • E MEDICINE